The genetic background of arrhythmogenic right ventricular cardiomyopathy

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The genetic background of arrhythmogenic right ventricular cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by degeneration of the right ventricle and ventricular tachycardia originating from the right ventricle. Additionally, the disease is an inherited cardiomyopathy that mainly follows the autosomal dominant pattern. More than 10 genes have been reported as causative genes for ARVC, and more than half of ARVC patients carry mu...

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Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heart muscle disease in which the pathological substrate is a fibro-fatty replacement of the right ventricular myocardium. The major clinical features are different types of arrhythmias with a left branch block pattern. ARVC shows autosomal dominant inheritance with incomplete penetrance. Recessive forms were also described, although i...

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Arrhythmogenic right ventricular cardiomyopathy (ARVC) has evolved from postmortem pathology at to a diagnosable clinical condition, and holds promise for definitive genetic diagnosis. Its prevalence is between 1/1,000 and 1/5,000, with 10% of deaths occurring before age 19 and 50% before age 35. When analyzed against age-specific norms, the electrocardiography (ECG) and signal-averaged ECG (SA...

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ژورنال

عنوان ژورنال: Journal of Arrhythmia

سال: 2016

ISSN: 1880-4276

DOI: 10.1016/j.joa.2016.01.006